1. [Hemoglobin C disease: report of 16 Tunisian cases].
- Author
-
Hafsia R, Marrakchi O, Ben Salah N, Gouider E, Ben Lakhal R, Jeddi R, Aissaoui L, Belhadjali Z, Ben Abid H, Meddeb B, and Hafsia A
- Subjects
- Adolescent, Adult, Female, Hemoglobin C analysis, Hemoglobin C Disease genetics, Humans, Hypersplenism etiology, Male, Middle Aged, Retrospective Studies, Splenomegaly etiology, Tunisia, Hemoglobin C Disease diagnosis
- Abstract
Aim: was to provide the clinical and biological patterns hemoglobine disease in Tunisia., Methods: This retrospective study collected to 16 cases of hemoglobin C disease : 6 homozygotic Hb C and 10 heterozygotic Hb C/beta-thalassemia., Results: The clinical profile is characterized by mild hemolytic anemia (Hb = 11.7 g/dl) associated with splenomegaly and hypersplenism. Contrary to homozygous state, the Hb C/beta-thalassemia is associated with microcytosis and pseudopolycythemia. The diagnosis is based on target cells, specific intraerythrocytic Hb C crystals in blood smear and Hb C level at 100%., Conclusion: The Hb C disease must be considered as a benign hemoglobinopathy which is associated with a long survival without major complications.
- Published
- 2007